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Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports
BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throu...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6686505/ https://www.ncbi.nlm.nih.gov/pubmed/31395055 http://dx.doi.org/10.1186/s12903-019-0872-8 |
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author | Kabali, Theodora Miti Moshy, Jeremiah Robert Owibingire, Sira Stanslaus Sohal, Karpal Singh Simon, Elison N. M. |
author_facet | Kabali, Theodora Miti Moshy, Jeremiah Robert Owibingire, Sira Stanslaus Sohal, Karpal Singh Simon, Elison N. M. |
author_sort | Kabali, Theodora Miti |
collection | PubMed |
description | BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment. |
format | Online Article Text |
id | pubmed-6686505 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-66865052019-08-12 Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports Kabali, Theodora Miti Moshy, Jeremiah Robert Owibingire, Sira Stanslaus Sohal, Karpal Singh Simon, Elison N. M. BMC Oral Health Case Report BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment. BioMed Central 2019-08-08 /pmc/articles/PMC6686505/ /pubmed/31395055 http://dx.doi.org/10.1186/s12903-019-0872-8 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Kabali, Theodora Miti Moshy, Jeremiah Robert Owibingire, Sira Stanslaus Sohal, Karpal Singh Simon, Elison N. M. Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title_full | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title_fullStr | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title_full_unstemmed | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title_short | Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports |
title_sort | craniofacial fibrous dysplasia associated with mccune-albright syndrome: challenges in diagnosis and treatment: case reports |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6686505/ https://www.ncbi.nlm.nih.gov/pubmed/31395055 http://dx.doi.org/10.1186/s12903-019-0872-8 |
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